Dementia Research Centre, UCL Institute of Neurology, University College London, Queen Square, London, UK.
Neurology. 2010 Dec 14;75(24):2204-11. doi: 10.1212/WNL.0b013e318202038c.
We sought to describe the antemortem clinical and neuroimaging features among patients with frontotemporal lobar degeneration with TDP-43 immunoreactive inclusions (FTLD-TDP).
Subjects were recruited from a consecutive series of patients with a primary neuropathologic diagnosis of FTLD-TDP and antemortem MRI. Twenty-eight patients met entry criteria: 9 with type 1, 5 with type 2, and 10 with type 3 FTLD-TDP. Four patients had too sparse FTLD-TDP pathology to be subtyped. Clinical, neuropsychological, and neuroimaging features of these cases were reviewed. Voxel-based morphometry was used to assess regional gray matter atrophy in relation to a group of 50 cognitively normal control subjects.
Clinical diagnosis varied between the groups: semantic dementia was only associated with type 1 pathology, whereas progressive nonfluent aphasia and corticobasal syndrome were only associated with type 3. Behavioral variant frontotemporal dementia and frontotemporal dementia with motor neuron disease were seen in type 2 or type 3 pathology. The neuroimaging analysis revealed distinct patterns of atrophy between the pathologic subtypes: type 1 was associated with asymmetric anterior temporal lobe atrophy (either left- or right-predominant) with involvement also of the orbitofrontal lobes and insulae; type 2 with relatively symmetric atrophy of the medial temporal, medial prefrontal, and orbitofrontal-insular cortices; and type 3 with asymmetric atrophy (either left- or right-predominant) involving more dorsal areas including frontal, temporal, and inferior parietal cortices as well as striatum and thalamus. No significant atrophy was seen among patients with too sparse pathology to be subtyped.
FTLD-TDP subtypes have distinct clinical and neuroimaging features, highlighting the relevance of FTLD-TDP subtyping to clinicopathologic correlation.
我们旨在描述具有 TDP-43 免疫反应性包含物的额颞叶变性患者的生前临床和神经影像学特征（FTLD-TDP）。
从一系列具有原发性神经病理学 FTLD-TDP 诊断和生前 MRI 的连续患者中招募了受试者。28 名患者符合纳入标准：9 名 1 型，5 名 2 型，10 名 3 型 FTLD-TDP。4 名患者的 FTLD-TDP 病理太少，无法进行亚型分类。回顾了这些病例的临床、神经心理学和神经影像学特征。使用基于体素的形态计量学评估与 50 名认知正常对照组的区域灰质萎缩。
临床诊断在各组之间有所不同：语义性痴呆仅与 1 型病理有关，而进行性非流利性失语症和皮质基底节综合征仅与 3 型有关。行为变异额颞叶痴呆和额颞叶痴呆伴运动神经元病在 2 型或 3 型病理中可见。神经影像学分析显示病理亚型之间存在明显的萎缩模式：1 型与不对称性前颞叶萎缩（左或右优势）有关，还涉及眶额叶和岛叶；2 型与内侧颞叶、内侧前额叶和眶额-岛叶皮质相对对称的萎缩有关；3 型与不对称性萎缩（左或右优势）有关，涉及更背侧的区域，包括额叶、颞叶和下顶叶皮层以及纹状体和丘脑。在病理分类太少的患者中未观察到明显的萎缩。
FTLD-TDP 亚型具有不同的临床和神经影像学特征，突出了 FTLD-TDP 亚型分类与临床病理相关性的重要性。
